Invasive extramammary Paget's disease of the vulva: incidence, trends and outcomes

Autor: Hsiu-Min Chen, Ashley Moon, Amandeep Mann, Ravali Reddy, John K. Chan, Cheng-I Liao, Daniel S. Kapp
Rok vydání: 2021
Předmět:
Zdroj: Gynecologic Oncology. 162:S192
ISSN: 0090-8258
DOI: 10.1016/s0090-8258(21)01014-3
Popis: Objectives: To explore the incidence, trends and survival outcomes of invasive extramammary vulvar Paget's disease compared to squamous and basal cell vulvar cancers. Methods: Incidence data were extrapolated from the United States Cancer Statistics (USCS) from 2001 to 2017 by SEER*Stat. Trends analysis was performed by Joinpoint regression program and presented by average annual percent change (AAPC). Clinical data on demographics, disease and treatment were recorded from the National Cancer Database from the same period. Chi-squared test, Kaplan-Meier survival estimates, and Cox proportional hazards model adjusted for covariates were used. Results: From 2001-2017, there were 3,715 cases of malignant extramammary Paget's disease of the vulva. Whites comprised 3,172 cases (85.4%), followed by Asian/Pacific Islanders with 227 cases (6.1%), Hispanics with 198 cases (5.3%), and Blacks with 44 cases (1.2%). Most cases had localized disease (73.1%), 10.4% had regional spread and 1.2% had distant disease. The overall incidence was 0.12/100,000 women per year in 2017. The incidence increased with an average percent increase of 0.4% per year from 2001 to 2017 (p Conclusions: Invasive extramammary Paget's disease of the vulva is uncommon though the incidence is increasing. Older age and advanced stage are associated with poorer survival. Patients with metastatic vulvar Paget's disease and squamous cell carcinoma have a poorer survival compared to metastatic vulvar basal cell carcinoma.
Databáze: OpenAIRE
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